Macrophage activation syndrome (MAS) is a rare immunological disorder, rapidly life-threatening in the absence of specific treatment. Malignant lymphoma is common cause of MAS, but Hodgkin's lymphoma has been rarely associated with MAS. We report a 79-year-old man who presented a MAS as the presenting feature of Hodgkin's lymphoma, with a rapidly fatal outcome. We discuss the usefulness of sCD25 and interleukine 18 assays when diagnosis is difficult to obtain. We also review the literature data related to Hodgkin's lymphoma-associated MAS.
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